Isolated noncompaction of the ventricular myocardium. A study in an adult male and literature review

Abstract

Noncompaction of the ventricular myocardium is a rare congenital disorder characterized by the presence of numerous prominent trabeculations and deep intertrabecular recesses which communicate with the left ventricular cavity. The disease uniformly affects the left ventricle, sometimes also affecting the right ventricle. Noncompaction of the ventricular myocardium is believed to be a disorder of endomyocardial embryogenesis. Familial occurrence has been observed. It may be accompanied by depressed ventricular function, cardiac arrhythmia and systemic embolism. Although noncompaction of the ventricular myocardium is a congenital myocardial disorder, the onset of symptoms is frequently delayed until adulthood. We describe a case of noncompaction of the ventricular myocardium in a 33-year-old male with the typical echocardiographic and cardiac magnetic resonance imaging features of this disease.